Evans综合征诊断和治疗中国专家共识(2024年版)  

Chinese expert consensus on the diagnosis and treatment of Evans syndrome(2024)

作　　者：中华医学会血液学分会红细胞疾病(贫血)学组 张连生[2] 邵宗鸿[3] 王化泉[3] 李莉娟[2] Red Blood Cell Disease(Anemia)Group,Chinese Society of Hematology,Chinese Medical Association;Zhang Liansheng;Shao Zonghong(不详;Department of Hematology,Lanzhou University Second Hospital,Lanzhou 730030,China;Department of Hematology,General Hospital,Tianjin Medical University,Tianjin 300052,China)

机构地区：[1]不详 [2]兰州大学第二医院,兰州730030 [3]天津医科大学总医院,天津300052

出　　处：《中华血液学杂志》2024年第9期809-815,共7页Chinese Journal of Hematology

摘　　要：Evans综合征(ES)是一种罕见的自身免疫性疾病,其特征是至少出现两种自身免疫性血细胞减少(AIC),AIC包括免疫性血小板减少症(ITP)、自身免疫性溶血性贫血(AIHA)和自身免疫性中性粒细胞减少症(AIN)。21%~50%的ES患者为继发性。本病有反复复发、可并发血栓及感染等严重并发症和高死亡率的特点。ES的治疗具有高度异质性,需兼顾AIC、原发疾病及并发症的治疗。目前国内外缺乏ES的前瞻性证据、随机对照试验,为进一步规范我国ES的诊治水平,中华医学会血液学分会红细胞疾病(贫血)学组参考国外ES诊治共识及国内外最新ES相关文献,制定本共识,旨在为临床ES的诊断和治疗提供参考意见。Evans syndrome(ES)is a rare autoimmune disorder characterized by the presence of at least two autoimmune cytopenias(AIC),including immune thrombocytopenia(ITP),autoimmune hemolytic anemia(AIHA),and autoimmune neutropenia(AIN).Secondary ES accounts for 21%-50% of cases.ES is characterized by recurrent relapses,serious complications such as thrombosis and infection,and high mortality.The management of ES is highly heterogeneous,necessitating treatment for AIC,the primary disease,as well as associated complications.However,there remains a lack of prospective evidence,randomized clinical trials for ES.To standardize the diagnosis and management of ES in China effectively,this consensus was developed by the Red Blood Cell Diseases(Anemia)Group under the Chinese Society of Hematology within the Chinese Medical Association.It aims to provide valuable guidance for diagnosing and managing ES in clinical practice based on international consensus and comprehensive review of both domestic and international literature.

关 键 词：EVANS综合征 中华医学会 中性粒细胞减少症 血细胞减少 自身免疫性疾病 中国专家共识 原发疾病 红细胞疾病 

分 类 号：R593.2[医药卫生—内科学]